Renal involvement in Waldenström’s macroglobulinemia: case report and review of literature

Waldenström’s macroglobulinemia (WM) is a rare lymphoid neoplasia, accounting for 2% of all hematological malignancies. Renal complications occur rather rarely compared to multiple myeloma. The most common renal manifestations are mild proteinuria and microhematuria. We describe a case of MW presenting with acute renal failure and NS. A 67-year-old man was referred to our hospital for sudden onset nephrotic syndrome. Electrophoresis revealed a monoclonal component in the gamma region, which was classified as an IgM k. During hospitalization, acute kidney injury developed, with creatinine up to 5?mg/dL, despite adequate hydration and alkalinization. A kidney biopsy was performed, showing minimal change disease (MCD) with interstitial and capsular lymphoid infiltrates of B-Lymphocytes CD20+. B-lymphocytes infiltration suggested the possibility of renal localization of lymphoproliferative disorder. So, bone marrow histology was performed, revealing lymphoplasmacytic lymphoma (WM). The patient was treated with bortezomib, desamethasone, and rituximab, with partial recovery of renal function (creatinine 1.5?mg/dL) and complete remission of proteinuria after 8-month follow-up. The remission of NS in our patient with rituximab seems to emphasize the pathogenetic role of B cells in MCD, although a coincident effect of immunosuppression on both the underlying renal disease and the hematologic disease cannot be excluded.     

Waldenström's macroglobulinemia and large B-cell central nervous system lymphoma: a case report

BACKGROUND: Waldenstr?m's macroglobulinemia is a low-grade B-cell lymphoma characterized by monoclonal synthesis and secretion of IgM antibodies [3]. The classic symptoms of WM result from blood hyperviscosity. Neurologic symptoms, such as fatigue, dizziness, and blurred vision, are common. The metastatic spread to the CNS, which rarely has been reported, can occur either diffusely or in the form of a mass lesion. CASE DESCRIPTION: We present the case of a 73-year-old woman with intracerebral large B-cell lymphoma and concurrent WM. CONCLUSION: This case illustrates the importance of histopathologic diagnosis by brain biopsy despite an existing diagnosis of systemic neoplasm.     

Cast nephropathy in a case of Waldenström's macroglobulinemia

Waldenstr?m's macroglobulinemia is a low-grade lymphoplasmacytic lymphoma characterized by a circulating monoclonal IgM. The clinical manifestations are due to deposition of IgM in liver, spleen, and/or lymph nodes. Related symptoms include anemia and complications of the hyperviscosity syndrome. Renal involvement in classical cases of Waldenstr?m's macroglobulinemia is rare, and the pathological hallmark finding in the renal biopsy specimen is a thrombotic microangiopathy. We report the case of a 73-year-old female with the diagnosis of pernicious anemia for 2 years before she presented with acute renal failure. A renal biopsy performed suggested the diagnosis of myeloma cast nephropathy. However, bone marrow biopsy specimens and hematological studies did not support this diagnosis. Serum and urinary protein electrophoresis revealed a monoclonal lambda subtype IgM. Ultrasound imaging showed an enlarged spleen. The diagnosis of cast nephropathy in a patient with Waldenstr?m's macroglobulinemia was made. She underwent treatment with fludarabine and plasmapheresis/hemodialysis with dramatic improvement of her renal function.     

Waldenström's macroglobulinemia and nephrotic syndrome with membranous nephropathy

Renal complications of Waldenstr?m's macroglobulinemia (WM) are rarely observed. Nephrotic syndrome in association with WM has most often been secondary to amyloidosis. This article reports a case of WM with nephrotic syndrome as a result of membranous nephropathy with immunoglobulin M (IgM) deposition. A 44-year-old male diagnosed with WM 4 years previously, presented with heavy proteinuria (7.8 g/24 h). Kidney biopsy revealed expanded mesangium, thickened capillary loops and epimembranous spikes, with no significant interstitial inflammation or thickened tubular basement membranes. Immunofluorescence examination demonstrated strong granular staining of IgM and λ chains, with weaker C3 and C1q staining. Electron microscopy showed many subepithelial dense deposits, and fewer large subendothelial dense deposits. Treatment was directed at the patient's WM with maintenance rituximab and fludarabine. Subsequently, decreases were seen in both the patient's serum IgM and serum viscosity. With therapy for WM and the addition of an angiotensin receptor blocker, the patient's proteinuria also improved, from 7.8 g to 4.8 g/24 h. The patient continued to follow up with his hematologist and in 2009 creatinine was 1 mg/dl (76.26 ?mol/l), with a 24 h urine protein excretion of 0.159 g.     

Pediatric Kienböck's disease: case report and review of the literature

A 13-year-old boy with symptomatic Stage III Kienb?ck's disease was treated successfully with a radial shortening procedure. A comparison of the preoperative and postoperative radiographs and magnetic resonance imaging studies showed evidence of lunate revascularization and remodeling after a radial shortening osteotomy.     

A case with acute renal failure complicated by Waldenström's macroglobulinemia and cryoglobulinemia

We encountered a 53-year-old man associated with acute renal failure caused by Waldenstr?m's macroglobulinemia and type I cryoglobulinemia. Treatment with prednisolone and cyclophosphamide induced a rapid recovery from acute renal failure. Renal histology revealed endocapillary proliferation and lobular formation with scattered subendothelial, amorphous and periodic acid-Schiff (PAS)-positive materials in the glomerular capillaries which were positive for IgM on immunofluorescence study. Although the exact mechanism for pathophysiology of acute renal failure remains unknown, treatment with prednisolone and cyclophosphamide could induce a rapid recovery from acute renal failure accompanied by Waldenstr?m's macroglobulinemia and type I cryoglobulinemia.     

Nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis in a patient of Waldenström's macroglobulinemia

A 72-year-old man was diagnosed as having nephritic syndrome complicated by Waldenstr?m's macroglobulinemia (WM). A monoclonal IgM lambda protein and decreased serum complements were observed. The renal biopsy disclosed the capillary occluded by thrombi which was stained with IgG, IgA, IgM, C4, lambda light chain and slight kappa light chain in a granular pattern. Electron dense deposits were noted in the subendothelial spaces. An unusual case of WM who developed nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis is described.     

Low-grade T-cell lymphoma of the kidney and Waldenström's macroglobulinemia in a patient presenting with renal failure

Renal failure is rarely the presenting manifestation of non-Hodgkin's lymphoma. We describe the unusual case of a patient who presented with uremia due to lymphomatous infiltration of the kidney by a low-grade T-cell lymphoma. The diagnosis of lymphoma was made by renal biopsy. Extrarenal nodular or extra-nodular involvement could not be detected. However, simultaneously, a lymphoplasmacytic lymphoma was found on bone marrow biopsy associated with IgM paraproteinemia. To our knowledge, this is the first report of a renal T-cell lymphoma associated with Waldenstr?m's macroglobulinemia.     

Henoch-Schönlein syndrome and cerebellar hemorrhage: report of an adolescent case and literature review

BACKGROUND: Henoch-Sch?nlein syndrome (HSS) is a systemic necrotizing vasculitis predominantly affecting children. Symptoms are usually self-limited and only rarely do they involve the central nervous system. Only five published reports describe cases of radiologically proven intracranial hemorrhages complicating HSS. CASE DESCRIPTION: In this 17-year-old boy, a cerebellar hemorrhage developed after aspecific symptoms of upper respiratory tract infection. His past medical history and emerging evidence of systemic bleeding yielded a diagnosis of recurrent HSS. This was the fourth time the disease had recurred since the age of 4. The patient underwent surgical treatment and returned to his normal activities. CONCLUSIONS: Intracerebral hemorrhages during HSS share a favorable prognosis and a posterior lobar localization, typically involving the parieto-occipital region. The case described here is unusual because the patient did not have the typical purpuric rash and unlike published cases, the intracranial hemorrhage marked the onset of HSS rather than complicating a typical HSS presentation.     

Bilateral Preiser’s disease: a case report and review of the literature

Avascular necrosis of bone may affect specific areas of the body where the blood supply to the bone or part of the bone is vulnerable to injury or disease. In the carpal scaphoid, it can find this entity consequent a trauma, microtrauma, or fractures, especially in the proximal pole (Preiser in Fortschr Geb Roentgenstr 15:189-197, 1910), associated with a systemic disease, steroid ingestion (Ferlic and Morin in J Hand Surg-A 14:13-16, 1989), chemotherapy (Green in Hand Clinic 3:163-168, 1987) or a hypoplastic scaphoid (Gunal et al., in J Hand Surg-B 20(6):736-740, 1995). Preiser's disease is a rare entity and is commonly associated with avascular necrosis of the scaphoid without fracture or trauma. A case of bilateral idiopathic avascular necrosis of the scaphoid is reported.     

Talonavicular synostosis with lateral ankle instability—A case report and review of the literature

Talonavicular coalition is a rare autosomal recessive congenital anomaly that is usually asymptomatic and detected incidentally on radiographs. It is associated with symphalangism, clinodactyly, a great toe that is shorter than the second toe, clubfoot, calcaneonavicular coalition, talocalcaneal coalition and a ball-and-socket ankle joint.The authors present a review of the literature and case report of a patient with complete osseous talonavicular coalition, talocalcaneal coalition and lateral ankle instability which was successfully treated with subtalar fusion and lateral ligament reconstruction.     

Post-Staphylococcal infection Henoch–Schönlein purpura nephritis: a case report and review of the literature

Abstract

A 37-year-old man developed Henoch--Schönlein purpura nephritis (HSPN) with nephrotic syndrome and rapidly progressive glomerulonephritis after otitis media and externa due to methicillin-resistant Staphylococcus aureus infection. Despite resolution of the infection and prednisolone therapy, his kidney disease worsened. However, the addition of cyclosporine A finally resulted in complete remission of the nephrotic syndrome. A review of similar cases with post-Staphylococcal infection HSPN revealed strong similarities between this entity and immunoglobulin A-dominant postinfectious glomerulonephritis (IgA-PIGN), an increasingly recognized form of PIGN typically associated with Staphylococcal infection, in both clinical and morphological features. Post-Staphylococcal infection HSPN may constitute a subgroup of IgA-PIGN.     

Adult Wilms’ tumor: A case report and review of literature

We report the case of a 43-year-old woman with adult Wilms’ tumor. Imaging studies confirmed the presence of a 25 × 20 × 12 cm mass in the left kidney. A radical transabdominal nephrectomy was performed. Histological diagnosis was adult Wilms’ tumor. Postoperative chemotherapy was offered to the patient who remains disease-free 67 months postoperatively. There are a few reported cases of this entity and till today the best treatment options and the prognosis remain unclear.     

Carcinosarcoma of esophagus—a case report and review of literature

Carcinosarcoma of esophagus contains both the carcinomatous and sarcomatous elements. These are rare polypoidal malignancies of esophagus. One such case is presented and available literature is reviewed.     

Rare double fracture-dislocation of the thumb： a case report and review of the literature

Simultaneous fracture/dislocation of the thumb carpometacarpal (CMC)joint and dislocation of the metacarpophalangeal (MCP)joint is considered as a rare injury pattern.We report an unusual case of dorsa...     

Vaginal repair of neobladder–vaginal fistula: a case report and review of the literature

Neobladder substitution after radical cystectomy for invasive cancer has become commonplace as it preserves normal volitional voiding through the native urethra and avoids urinary diversion. Neobladder–vaginal fistula (NVF) is a rare postoperative complication of this procedure that results in decreased quality of life. We describe a case of vaginal repair of a NVF and present a review of the literature.     

Cutaneous melanoma with neurofibromatosis type 1: rare association? A case report and review of the literature

Neurofibromatosis (NF) is a relatively common disorder characterized by cutaneous pigmented maculas, multiple neurofibromas and Lisch nodules (pigmented iris hamartomas). This disorder is retained being a neurocristopathy. Melanocytes are neural crest derivates too. Twenty-six patients with neurofibromatosis associated to cutaneous malignant melanoma have been reported till now, but data on association between these two pathologies are lacking. One more case of malignant cutaneous melanoma in a patient with neurofibromatosis is reported and the hypothesis of a more frequent association than usually believed of these two pathologies is discussed.